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题目:Modeling inherited metabolic disorders of the liver using human induced pluripotent stem cells! n* p+ A O) Y r1 Z0 f
发表在著名杂志JCI(Journal of Clinical Investigation,IF=15.387)上,最新文章!6 P; K& r/ a, q. P1 W
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本文章1.不仅从α1-antitrypsin deficiency,familial hypercholesterolemia, and glycogen storage disease type 1a这三种肝病的病人诱导出iPS;2.还有效分化:Each line was differentiated into hepatocytes using what we believe to be a novel 3-step differentiation protocol in chemically defined conditions;3.且最后模拟并重现了疾病情况:recapitulate key pathological features of the diseases affecting the patients from which they were derived, such as aggregation of misfolded α1-antitrypsin in the endoplasmic reticulum, deficient LDL receptor–mediated cholesterol& {: x8 A3 U% v' {$ o8 O P* y# i
uptake, and elevated lipid and glycogen accumulation.( E# `4 T& `9 G3 H' X
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